• Childhood Kidney Diseases
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• v.1 no.2
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• pp.170-175
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• 1997

Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.92-101
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• 2000

Purpose : We analyzed the demogaphic data md clinical course of Korean children with chronic renal failure (CRF) observed between 1990 and 1999. Patients and Methods : Questionnaires were mailed to all children's hospitals ail through the country. We asked for primary renal disease age and serum creatinine levels at first presentation with CRF and end-stage renal disease (ESRD), and modes of renal replacement therapy (RRT). Results : 401 children (254 boys, 147 girls) with CRF, defined as a permanent increase of serum creatinine above 1.2 mg/dl for at least 3 months or until death, were identified. This represents an incidence of 3.68 per million child population per year. Of these patients, 22$\%$ on younger than 5 years, 28$\%$ 5 to 10 years and 50$\%$ 10 to 15 year. Eight five $\%$ of the patients could be classified with a primary renal disease. The most frequent cause is glomerulonephritis (36$\%$), followed by chronic pyelonephritis (21$\%$), renal hrpo/dylplasia (9$\%$), and hereditary nephropathies (7$\%$). Reflux nephropathy (16$\%$) was the most common single cause of CRF. ESRD was reached in 70$\%$ of all patient. 99.3$\%$ of these started RRT. Hemodialysis (HD, 42$\%$), peritoneal dialysis (PD, 35$\%$) and transplantation (TP, 23$\%$) were performed as the initial mode of RRT. A total of 161 TPs were performed (159 first grafts, 2 second grafts). A total of 32 patients died. The main causes of death were dialysis related complication in HD patients and infections in PD patients. Survival rate on any form of RRT was 88.7$\%$ during the mean follow-up period of 37 months. Conclusion Major efforts should be directed toward earlier diagnosis and treatment of reflux nephropathy to prevent occurrence of Of. Dialysis and TP have now become well accepted forms of treatment in Korean children with ESRD.

• Childhood Kidney Diseases
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• v.4 no.2
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• pp.102-110
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• 2000

Purpose : Intravenous infusion of albumin has been widely und to relieve severe nephrotic edema in spite of beneficial or harmful effects. The purpose of this study is to examine the harmful effect of albumin on the clinical course of steroid sensitive nephrotic syndrome(SSNS). Method : We prospectively randomized the patients with nephrotic syndrome(biopsy proven or clinically compatible to minimal change nephrotic syndrome) into the albumin group(20$\%$ albumin 1 g/kg) or control group(5$\%$ D/W) between March 1997 and September 1999 at Ewha University Mokdong Hospital. We compared the clinical course of the albumin group(n=13) with the control group(n=13). Results : 1. The duration of steroid therapy until complete remission in the albumin group was significantly longer than the control group($13.7{\pm}6.4\;days\;vs\;7.5{\pm}2.8\;days$)(P<0.05). 2. The remission duration to the first relapse was significantly shorter in the albumin group ($94{\pm}63.5$ days) than the control group($190{\pm}106.4$ days)(P< 0.05). There was no significant difference in the relapse rate within 1 year after complete remission[77$\%$ (10/13) vs 46$\%$ (6/13)](P>0.05) and the relapse frequency per year($1.9{\pm}0.8\;vs\;1.5{\pm}0.5$) between the albumin and control groups. 3. Spot urine protein/creatinine ratio significantly increased in the albumin group at post-albumin 2, 4 days(P<0.05). There was no significant difference in the change of the daily percent weight loss, blood pressure, serum Na, K concentration between the albumin and control groups. Conclusion : Albumin infusion in SSNS delayed the response to steroid and shortened the remission duration to the first relapse. Albumin should be carefully used in nephrotic edema.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.155-160
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• 1997

Purpose : Tethered cord syndrome is characterized by progressive motor and sensory disturbances in lower extremities, foot deformities caused by a pathologic fixation of spinal cord resulting in excessive stretching of the spinal cord. It is also frequently associated with urological symptoms include urinary frequency, incontinence, enuresis, urgency and recurrent urinary tract infection. Because there is few report in the literature about urological manifestations of theterd cord syndrome, we conducted a retrospective study on the patients diagnosed as tethered cord syndrome to delineate the characteristics of urologic manifestations in tethered cord syndrome and to establish the policy to evaluate patients who is suspected of tethered cord syndrome. Method : A retrospective study was conducted by reviewing the medical records of nine patients who was diagnosed as tethered cord syndrome from November 1991 to July 1996 in Yeungnam University Hospital. Result : 1) The age distribution of nine patients was as follows; 5 patients were under 2 years, 1 case from 2 to 6 years and 3 cases from 6 to 10 years. 2) Of 9 patients 6 had voiding frequency, urinary incontinence, enuresis, urgency and loss of micturation sense. Radiologic urodynamic studies revealed neurogenic bladder in 5 patients, hydronephrosis and hydroureter in 3, vesicoureteral reflux in 3. 3) Of 6 patients with urological abnormal manifestations 4 underwent spinal cord surgery (detethering). In spite of surgical intervention, the urological manifestations improved in only one patient. Conclusion : Urological abnormalities were common in tethered cord syndrome. Abnormal laboratory findings including urodynamic study were found even before the onset of urological symptoms. We have to concern tethered cord syndrome as one of common causes of voiding frequency and enuresis and to evaluate urological abnormalities as thetered cord syndrome is diagnosed.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.109-116
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• 1997

Purpose : Chronic renal failure is often accompanied by severe dyslipidemia, a known risk factor for cardiovascular disease. Lipoprotein(a) [Lp(a)] has recently been characterized as a risk factor for atherosclerosis and thrombosis. Cardiovascular disease is the leading cause of death in adult patients on dialysis. However, there are only limited data available concerning risk factors for atherosclerosis in uremic children. We have measured serum levels of lipids, lipoproteins, apolipoproteins and Lp(a) in uremic children with maintenance dialysis. Methods : Ten uremic children with hemodialysis (HD) and 14 with peritoneal dialysis (PD) in our dialysis unit were included in this study. The mean age of HD patients was $162{\pm}59$ months and the male to female ratio was 7:3. The mean age and sex ratio of PD patients were $123{\pm}63$ months and 6:8, respectively. The levels of cholesterol, triglyceride, lipoproteins, apolipoproteins and Lp(a) were measured from serum sampled after 14 hours of fasting. The normal control levels were cited from 2 articles presenting the normal blood lipid and lipoprotein levels of primary school and middle school children in Korea. Results : There was no difference in age, sex ratio, body mass index and duration of dialysis between the HD and the PD group. The serum concentration of the cholesterol, triglyceride and apolipoprotein B were significantly elevated in dialysis patients compared with normal subjects. The serum level of Lp(a) was significantly elevated in only PD group. The serum Lp(a) level was below 30 mg/dl in 13 and above 30 mg/dl in 11 patients. The serum albumin level was significantly decreased in high Lp(a) group than in low Lp(a) group. Conclusion : The uremic children receiving dialysis reveal abnormal serum lipid and lipoprotein profiles. These results suggest that they have a higher risk for coronary heart disease, although there has been no clinical evidence of coronary heart disease at present. A long-term follow-up study of these children to clarify the suggestion should be started now.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.220-228
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• 2007

Purpose : We studied the value of clinical signs, laboratory findings and $^{99m}technetium$ dime-rcaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). Methods : A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. Results : Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. Conclusion : An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. There-fore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.195-202
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• 2007

Purpose : The normal flora, which was suggested to prevent infection, is acquired first from the birth canal and develops by dietary factors. Here presents a case-control study, aimed to evaluate the postnatal acquisition factors relating to the achievement of the normal flora in infants with urinary tract infection (UTI). Methods : 115 UTI infants, admitted at Ewha Womans University Mokdong Hospital from 2004 to 2005 and 116 age-matched control infants, who had visited well baby clinic, were evaluated. The suggested postnatal acquisition factors of the normal flora such as vaginal delivery, breast feeding, probiotics and yogurt intake and their relationship with UTI were evaluated. Results : The rate of vaginal delivery was 50%(58/l15) in UTI infants, which was not significantly different from 60%(69/116) in control infants(P>0.05). In the infants with UTI, the feeding pattern(breast milk 19%, mixed 26%, formula 55%) was significantly different from that(44%, 19%, 37%) in control infants(P<0.05). This significant difference was shown only in infants less than 6 months of age, but was not in infants over 6 months of age. The rate of probiotics intake in UTI infants was 4%(4/115), which was significantly lower than 27% (32/116) in control infants(P<0.05). The rate of regular intake of yogurt in UTI infants over 6 months of age was 27%(6/23), which was not significantly different from 35%(8/23) in control infants(P>0.05). The odds ratios of breast feeding and probiotics intake against UTI were significantly low as 0.30 (95% Cl 0.17-0.55)(P<0.01) and 0.03 (95% Cl 0.01-0.07)(P<0.01). Conclusion : The significantly lower rates of breast feeding and probiotics intake in UTI infants suggest that these dietary factors might have preventive effects in infants with UTI.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.272-279
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• 2007

Purpose : We tried to find out the clinical parameters which predict the outcome of treatment in children with enuresis. Methods : Enuresis patients who visited our hospital during 2003-2007 were included. Parameters such as age, gender, height, weight, minimal voided volume, maximal voided volume, maximum functional bladder capacity, frequency of voiding, urine S,G. before and after sleep were measured and an enuresis diary was also recorded. The reduction in wetting frequencies were classified into three groups; none(<50%), partial(50-90%) and complete(90%) response groups. We also compared the 'initial responders' who showed improvement(${\ge}50%$) during the 2 weeks of evaluation and behavioral therapy to the 'initial non-responders'. Results : Parameters mentioned above showed no significant relation to the treatment out-come. The response rate during the 2 weeks of the evaluation period was 32%(49/151) [complete in 1.3% (2/151), partial in 29.6% (47/151)]. Two-months' treatment responses were complete in 14(40%), partial in 19(54.3%) and none in 2(5.9%) responders(n=35), while they were 10(13.5%), 46(62.2%) and 18(24.3%), respectively in the non-responders(n=73) (P<0.05). Conclusion : We suggest that initial 'responsiveness' can be used as a predictor for good treatment outcome in patients with enuresis.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.255-263
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• 2007

Purpose : A single center cross sectional retrospective study was performed to compare the outcomes of different peritoneal dialysis(PD) modalities in Korean children. Methods : Among children dialyzed with PD between the year 2004 and 2007, 35 children had reliable data on PD adequacy after 3 to 15 months of dialysis. Subjects were grouped by their modalities; 17, 13 and 5 children were on continuous ambulatory PD(CAPD), continuous cyclic PD(CCPD) and nightly intermittent PD(NIPD), respectively. Body weight and height, number of patients taking anti-hypertensives and laboratory data including biochemical and hemoglobin levels were compared. Dialysis adequacy including weekly Kt/Vurea, creatinine clearance (Ccr) and daily water removal were also compared. Patients were sub-grouped by their peritoneal permeability characteristics. Results : The percentage of patients taking anti-hypertensives, monthly change in Z-scores of body weight and height and laboratory data did not differ among the groups. Patients on CAPD and CCPD showed similar dialysis adequacies. Weekly dialytic Ccr was significantly lower in the NIPD group compared to the others. But total Ccr was not different when residual renal function was added. Weekly dialytic Ccr by CAPD was significantly higher than that of CCPD in low and low-average transporters. Conclusion : We propose that modality can be selected flexibly according to the patients' preferences. And peritoneal permeability characteristics provide valuable information for adjusting PD prescriptions in ultrafiltration failure or in inadequate dialysis. Further study of other clinical performance measures should be performed to clarify the comparable outcomes in different PD modalities.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.77-84
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• 2014

Purpose: The purpose of this study was to compare the validity of Onen's alternative grading system (AGS) with that of the APDRP and SFU grading systems in patients with isolated and complicated congenital hydronephrosis. Methods: We retrospectively reviewed the medical records of 153 patients (204 renal units) diagnosed with congenital hydronephrosis between January 2002 and December 2011. We classified patients into 2 groups; isolated or complicated hydronephrosis. All renal units were graded according to anterior-posterior diameter of renal pelvis (APDRP), Society for Fetus Urology (SFU) and Onen's grading systems. We analyzed the prognosis of hydronephrosis, according to each grading system, at 2 years of age. Results: There were 152 renal units with isolated hydronephrosis and 52 renal units with complicated hydronephrosis. The isolated hydronephrosis group had a lower grade according to Onen's AGS, and showed more frequent spontaneous remission by 2 years of age. There was more frequent obstruction (P =0.000) and surgical treatment (P =0.000) of units with high-grade hydronephrosis according to Onen's AGS. In the complicated hydronephrosis group the frequencies of spontaneous remission (P =0.015) and renal dysfunction (P =0.013) were significantly higher than those in high-grade hydronephrosis, as indicated by Onen's AGS. There were no significant differences in clinical outcomes among the highest grade groups, according to the 3 systems, in either isolated or complicated hydronephrosis. Conclusion: Onen's AGS reflects the prognosis of hydronephrosis as well as other grading systems in those with isolated hydronephrosis. It was better predictor of renal dysfunction in those with complicated hydronephrosis. However, Onen's AGS was not superior to the other grading systems in terms of predicting prognosis, especially in high-grade hydronephrosis.